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Block 10 Week 4 > Usera: Liver 2 > Flashcards

Usera: Liver 2 Flashcards

1
Q

Idiopathic, chronic progressive hepatitis
Female preponderance
Defective t-cell regulation
May be triggered by infection, acute illness, drugs
a/w other Autoimmune disease
Portal plasma cell infiltrate
Elevated serum IgG and γ-globulin levels

A

autoimmune hepatitis

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2
Q

Which antibodies are associated with type 1 autoimmune hepatitis? What serotype is type 1 associated with?

A

Anti-nuclear antibodies (ANA)
Anti-smooth muscle antibodies (SMA)
Anti-actin antibodies (AAA)
Anti-soluble liver antigen/liver-pancreas antigen antibodies (SLA/LP)

associated with HLA-DR3 serotype

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3
Q

Which antibodies are associated with type 2 autoimmune hepatitis?

A

Anti-liver kidney microsome-1 antibodies (ALKM-1)
Directed against CYP2D6
Anti-liver cytosol-1 antibodies (ACL-1)

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4
Q

(blank) is the most common cause of fulminant hepatitis

A

toxicity

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5
Q

What are three mechanisms of toxin damage in the liver?

A

direct injury
injury due to toxic metabolites (ex: reactive intermediate of acetaminophen)
immunogenic

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6
Q

What can acetaminophen toxicity due to the liver?

A

causes perivenular necrosis

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7
Q

What kind of drugs can cause cholestasis?

A

Contraceptives, anabolic steroids, estrogen replacement therapy

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8
Q

What kinds of drugs can cause cholestatic hepatitis?

A

antibiotics, phenothiazines

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9
Q

Rare and potentially fatal syndrome of mitochondrial dysfunction in liver and brain
Characterized by extensive microvesicular steatosis
Associated with administration of acetylsalicyclic acid (aspirin)
Avoid use of aspirin in children

A

Reye syndrome

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10
Q

It is important to avoid the use of (blank) in children to avoid Reye’s syndrome (mitochondrial dysfunction in liver & brain)

A

aspirin

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11
Q

Appears acutely after heavy drinking episode
Lab findings may range from minimal to fulminant hepatitis
Anorexia
Weight loss
Upper abdominal discomfort
Tender hepatomegaly

A

alcoholic steatohepatitis

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12
Q

Final and irreversible form of alcoholic liver disease

A

alcoholic cirrhosis

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13
Q

T/F: Only 10-15% of patients with alcoholic liver disease develop cirrhosis

A

True

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14
Q

What causes fatty changes of microvesicular & macrovesicular steatosis?

A

alcohol!

**fatty change is reversible with abstention from alcohol

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15
Q

What are the clinical features of fatty liver?

A

mild elevation of serum bilirubin

mild elevation of alkaline phosphatase

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16
Q

What are some histologic features of alcoholic steatohepatitis?

A

hepatocytes swelling (ballooning degeneration)
mallory bodies
lymphocyte & neutrophilic inflammation
perisinusoidal fibrosis

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17
Q

This is an acquired disorder of hepatic metabolism

A

non-alcoholic fatty liver disease

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18
Q

These are inherited disorders of hepatic metabolism

A

hemochromatosis
Wilson disease
alpha-1 anti-trypsin deficiency

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19
Q

Most common cause of chronic liver disease in the US

70% of obese individuals have some form

A

non-alcoholic fatty liver disease

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20
Q

These are liver conditions seen in people who do not consume much alcohol

A

Hepatic steatosis
Steatosis with minor inflammation
Non-alcoholic steatohepatitis (NASH)

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21
Q

T/F: Hepatic steatosis with or without inflammation is a stable condition without significant clinical problems

A

True

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22
Q

Non-alcoholic fatty liver disease is strongly associated with (blank) and (blank)

A

obesity

metabolic syndrome

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23
Q

Homozygous recessive inherited disorder of excessive body iron absorption

A

primary hemochromatosis

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24
Q

Iron accumulation (hemosiderosis) is due to acquired causes

A

secondary hemochromatosis

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25
Q

Hereditary hemochromatosis can be associated with mutations in the following…

A

HFE
Transferrin receptor 2
Hepcidin genes
HJV gene

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26
Q

What is the normal total body iron? How much iron must have accumulated before disease will manifest (hemochromatosis)?

A

total body iron: 2-6GM

Disease will manifest after 20 gm stored have accumulated

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27
Q

How does iron damage liver tissues?

A

it is directly toxic to the tissues
lipid peroxidation thru Fe-catalyzed free radical production
Stimulation of collagen formation through activation of hepatic stellate cells
interaction of ROS & Fe with DNA leads to lethal cell injury

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28
Q

Are the toxic effects of iron to liver cells reversible?

A

yes, if the cells are not fatally injured

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29
Q

Main regulator of Fe absorption by lowering plasma Fe levels
Functions through an efflux channel ferroportin that prevents release of Fe from intestinal cells and macrophages
A deficiency causes iron overload

A

Hepcidin

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30
Q

Iron overload can be due to deficiency in this iron regulator

A

hepcidin

**hepcidin keeps iron inside of cells

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31
Q

Regulates hepcidin levels

Mutations cause severe juvenile hemochromatosis

A

HJV (hemojuvelin)

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32
Q

Regulates hepcidin levels

Mutations cause classic adult hemochromatosis

A

transferrin receptor 2

or

HFE (hemochromatosis gene)

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33
Q

Who gets hemochromatosis? What are the symptoms?

A 
male predominance
hepatosplenomegaly
abdominal pain
skin pigmentation
diabetes mellitus (due to pancreatic fibrosis)
cardiac dysfunction
arthritis
hypogonadism
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34
Q

Disease of unknown origin manifested by severe liver disease and extrahepatic hemosiderin deposition
Not an inherited disease
Liver injury occurs in utero possibly due to maternal alloimmune injury to fetal liver

A

neonatal hemochromatosis

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35
Q

Autosomal recessive disorder caused by a mutation in the ATP7B gene
There is impaired copper excretion into bile
Failure to incorporate copper into ceruloplasmin

A

Wilson disease

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36
Q

Where does copper accumulate in Wilson disease? What are the manifestations?

A

liver –> steatosis, hepatitis, cirrhosis
brain –> atrophy of basal ganglia & putamen
eye –> Kayser-Fleischer rings

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37
Q

What does a mutation in ATP7B cause?

A

decreased copper transport into bile
impaired copper incorporation into ceruloplasmin
inhibition of ceruloplasmin secretion into blood

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38
Q

How can you diagnose Wilson disease?

A

Serum ceruloplasmin levels
Increase in hepatic copper content
Increased urinary excretion of copper
**Do not use serum copper levels

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39
Q

What are the clinical features of Wilson disease?

A

chronic liver disease

neuropsych manifestations

40
Q

Autosomal recessive disorder that is characterized by decreased inhibition of proteases
Manifests as
Pulmonary emphysema
Liver disease

A

Alpha-1 antitrypsin deficiency

41
Q

Synthesized in the liver
A serine protease inhibitor
Polymorphic with at least 75 forms identified

A

alpha-1 antitrypsin

42
Q

What are the common genotypes of alpha-1 antitrypsin

A

PIMM (normal)
PIZZ (clinically significant mutation)
PIMZ (decreased alpha-1 antitrypsin)

43
Q

In what disease state will you see PASD+ intracellular accumulations?

A

alpha-1 antitrypsin deficiency

44
Q

Due to uncorrected obstruction of the extrahepatic biliary tree
Extrahepatic cholelithiasis
Malignancy of the biliary tree or head the pancreas
Strictures from previous surgical procedures
Biliary atresia
Cystic fibrosis
Choledochal cysts
Paucity of bile duct syndromes

A

Secondary biliary cirrhosis

45
Q

What happens in secondary biliary cirrhosis?

A

initially, there is cholestasis which is reversible with the correction of the obstruction
then, secondary inflammation initiates periportal fibrosis –> leads to hepatic scarring & nodule formation

46
Q

Inflammatory autoimmune disease affecting the intrahepatic bile ducts
Primary feature is a nonsuppurative inflammatory destruction of medium- and small sized intrahepatic bile ducts
Antimitochondrial antibodies are characteristic and essential for the diagnosis

A

primary biliary cirrhosis

47
Q

What is the primary feature of primary biliary cirrhosis?

A

inflammatory destruction of medium & small sized intrahepatic bile ducts

48
Q

What is essential for the diagnosis of primary biliary cirrhosis?

A

antimitochondrial antibodies

49
Q

What are the clinical features of primary biliary cirrhosis?

A

usu in middle aged women
insidious onset
fatigue & abdominal discomfort

50
Q

Chronic cholestatic disorder characterized by non-specific inflammation, sclerosing fibrosis and strictures of the large intra and extra hepatic bile ducts
May result from immunologically mediated injury

A

primary sclerosing cholangitis

51
Q

Primary sclerosing cholangitis involves sclerosing fibrosis & strictures of the (blank) and is associated with (blank)

A

large intra/extra hepatic bile ducts; ulcerative colitis

52
Q

Small clusters of modestly dilated bile ducts embedded in fibrous stroma
AKA “bile duct hamartomas”
Common and clinically insignificant
Associated with PCKD

A

Von Meyenberg complexes

53
Q

Multiple diffuse cysts in the liver

Associated with PCKD

A

Polycystic liver disease

54
Q 
Arises due to a persistence of the embryonic form of the biliary tree 
Portal tracts show fibrosis
Normally no cirrhosis
Increased risk of cholangiocarcinoma 
Associated with PCKD
A

Congenital hepatic fibrosis – a/w Caroli’s disease

55
Q

Larger ducts of the intrahepatic biliary tree are segmentally dilated
Associated with congenital hepatic fibrosis
Increased risk of cholangiocarcinoma
Associated with PCKD

A

Caroli disease

56
Q

Congenital absence/dearth of bile ducts

Rare autosomal dominant multi-organ disorder

A

Alagille syndrome

57
Q

What are 5 major clinical features of congenital absence of the bile ducts (Alagille syndrome)?

A 
Chronic cholestasis
Peripheral stenosis of the pulmonary artery
Butterfly-like vertebral arch defects
Eye defects
Peculiar hypertelic facies
58
Q

Pseudo-mass lesion in an otherwise normal liver
Central stellate scar from which fibrous septa radiate
Results from congenital vascular malformation

A

Focal nodular hyperplasia

59
Q

What is the major notable feature in a focal nodular hyperplasia?

A

central stellate scar from which fibrous septa radiate

60
Q

Most common benign liver tumor
Tumor of vascular channels in a bed of fibrous connective tissue
Should not be mistaken for metastatic tumors
Blind percutaneous biopsies should not be performed

A

cavernous hemangioma

61
Q

Is a cavernous hemangioma benign or malignant?

A

benign!

62
Q

Benign tumor arising from hepatocytes
Also called liver cell adenoma
Strongly associated with oral contraceptives or anabolic steroid use
Subcapsular adenomas may rupture causing severe intra-abdominal hemorrhage

A

hepatic adenoma

63
Q

Hepatic adenomas are highly associated with use of ?

A

oral contraceptives

anabolic steroids

64
Q

What can happen to subcapsular adenomas in the liver?

A

they can rupture leading to severe intra-abdominal hemorrhage

65
Q

Malignant tumor of young children, usually fatal within a few years
Two types
Epithelial type that recapitulates the developing liver
Mixed epithelial and mesenchymal types that shows areas of primitive mesenchyme
May be associated with familial adenomatous polyposis syndrome of Beckwith-Wiedmann syndrome

A

hepatoblastoma

66
Q

Two types of hepatoblastoma?

A

epithelial type: looks like the developing liver

mixed epithelial & mesenchymal type: shows areas of primitive mesenchyme

67
Q

Who get hepatoblastomas? Benign or malignant?

A

malignant tumor of young children

68
Q

Accounts for more than 90% of primary liver cancers

A

hepatocellular carcinoma

69
Q

List four things that can cause hepatocellular carcinoma

A

chronic viral infection
chronic alcoholism
non-alcoholic steatohepatitis
food contaminants

70
Q

What are the three types of hepatocellular carcinoma?

A

uninodular
multinodular
diffusely infiltrative

71
Q

Hepatocellular carcinomas have a strong propensity to invade (blank)

A

vascular structures, like the portal vein & IVC

72
Q

This is a variant of hepatocellular carcinoma that occurs in young males & females with NO underlying chronic liver disease or cirrhosis
Serum shows elevated AFP levels

A

fibrolamellar variant

73
Q

Malignancy of the biliary tree arising from bile ducts within and outside the liver

A

cholangiocarcinoma

74
Q

What are risk factors for cholangiocarcinoma?

A

primary sclerosing cholangitis
congential fibropolycystic disease
HCV infection
thorotrast

75
Q

Extrahepatic perihilar tumors (near the formation of the common hepatic duct) are known as (blank) tumors

A

Klatskin

76
Q

Most common sarcoma arising in the liver
Very aggressive malignant tumor with widespread metastasis
Thorotrast, vinyl chloride and arsenic exposure have been implicated as causative

A

angiosarcoma

77
Q

What causes angiosarcomas?

A

thorotrast
vinyl chloride
arsenic exposure

78
Q

Mets are far more common than primary hepatic neoplasia. Most common primary sites for mets?

A

colon
breast
lung
pancreas

79
Q

What are the 3 major groupings of circulatory disorders?

A

Impaired blood into the liver
Impaired blood flow through the liver
Hepatic venous outflow obstruction

80
Q

What can cause impaired blood inflow into the liver?

A

hepatic artery compromise - due to embolism, neoplasia, polyarteritis nodosa or sepsis

portal vein obstruction & thrombosis

81
Q

What are some symptoms of portal vein obstruction & thrombosis?

A

abdominal pain
portal hypertension
ascites

82
Q

What kinds of things can cause impaired blood flow through the liver?

A 
Cirrhosis
Sickle cell disease
DIC
Metastatic tumor
Eclampsia
Right and left sided heart failure
Peliosis hepatis
83
Q 
Thrombosis of 2 or more major hepatic veins
Produces liver enlargement, pain, ascites
¾ of patients have predisposing factors 
Hypercoagulable state 
Polycythemia vera
Factor V Leiden mutation
Contraceptive use
Pregnancy
A

Budd-Chiari syndrome

84
Q

Wide range of presenting symptoms (hepatic dysfunction, hepatic failure, coma, death)
Mitochondrial dysfunction implicated as the cause
Primary treatment is termination of pregnancy

A

acute fatty liver of pregnancy

85
Q

Altered hormonal state of pregnancy with biliary defects in secretion creates cholestasis
Benign clinical course

A

intrahepatic cholestasis of pregnancy

86
Q

What is cholelithiasis?

A

gallstones

87
Q

What are the two types of gallstones?

A

cholesterol stones

pigment stones - composed of bilirubin calcium salts

88
Q

What is cholecystitis?

A

inflammation of the gallbladder

89
Q

Calculous acute cholecystitis is due to (blank) from obstruction of the neck or cystic duct; acalculous is due to (blank)

A

chemical irritation & inflammation; ischemia

90
Q

Clinical features of acute cholecystitis?

A 
RUQ or epigastric pain
fever
anorexia
tachycardia
nausea
vomiting
91
Q

Clinical features of chronic cholecystitis?

A

Recurrent attacks of steady or colicky epigastric or RUQ pain
Nausea
Vomiting
Intolerance for fatty foods

92
Q

Complete or partial obstruction of the lumen of the extrahepatic biliary tree within the first 3 months of life

A

Biliary atresia

93
Q

What happens in biliary atresia

A

complete or partial obstruction of the biliary tree causes progressive inflammation and fibrosis of intrahepatic and extrahepatic bile ducts

94
Q

Two forms of biliary atreisa

A

Fetal: aberrant intrauterine development of the extrahepatic biliary tree
Perinatal: normally formed biliary tree is destroyed following birth

95
Q

Congenital dilations of the common bile duct
Patients present with jaundice or biliary colic
Predisposed to stone formation, stenosis and stricture, pancreatitis and obstructive biliary complications

A

choledochal cysts

Block 10 Week 4 (8 decks)

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